PB2421: EPSTEIN-BARR VIRUS-ASSOCIATED POST-TRANSPLANT LYMPHOPROLIFERATIVE DISEASE: RESULTS OF A DELPHI STUDY IN SPAIN.
Authors: Anna Sureda, Perez Simon Josè Antonio, Hesse Cristina
Journal: HemaSphere (2023)

Topic: 22. Stem cell transplantation - Clinical Background: Epstein-Barr virus positive post-transplant lymphoproliferative disorder (EBV+PTLD) is a rare disease that can occur in immunosuppressed patients after allogeneic hematopoietic cell transplantation (HCT) or solid organ transplantation (SOT). This disease has a very severe prognosis in patients who fail first line of treatment. In Spain, the current situation and management of patients with EBV+PTLD is not well-known. Aims: The objective of the study is to determine, in Spain, the burden of disease, patient management and unmet needs of patients with relapsed or refractory Epstein-Barr virus positive post-transplant lymphoproliferative disease (EBV+ PTLD) who have received at least one prior therapy. Methods: The study has been developed using Delphi methodology in two rounds of consultation with 12 hematologists with experience in the management of EBV+PTLD, who reported their opinion and experience of their clinical practice. Results: The estimated incidence of EBV+PTLD in Spain is of about 1.8% of the HCT and 1.7% of the SOT. For patients after HCT, approximately 100% are EBV+ and 50% are R/R after the first line of treatment. For SOT, 50% are EBV+ and 33% fail first-line treatment. Overall, it is estimated that approximately 9-47 patients are diagnosed with R/R EBV+PTLD after HCT and SOT in Spain each year. All patients require hospitalization, although they rarely visit the ICU (the decision is made on an individual basis). Hematologists are primarily responsible for the diagnosis, management, treatment and follow-up of these patients. Other professionals involved in the management of the patient are the SOT specialist, intensive care medicine, infectious diseases and the oncologist. All experts analyze the EBV viral load and use imaging techniques (PET/CT) for diagnosis. Nodal biopsy is the confirmatory diagnostic technique. Hematologists use different clinical guidelines for the management of EBV+PTLD. The clinical guidelines most widely used are: GELTAMO, ECIL-6 and NCCN respectively. The treatment of EBV+PTLD in clinical practice is based on the reduction of immunosuppression and the administration of rituximab and chemotherapy. After failure of these alternatives, patients’ options are extremely limited. After treatment, the main techniques used during follow-up of patients include the analysis of EBV viral load and the use of imaging techniques (PET/CT). The main unmet needs in the treatment of EBV+PTLD in Spain are the availability of an effective and safe treatment in terms of survival, response and tolerability, the need for multidisciplinary management and the need to raise awareness about this ultra-rare condition. Summary/Conclusion: This study provides a description of the current situation of patients with R/R EBV+PTLD in Spain. The main unmet need identified by hematologists in Spain is the lack of an effective and safe treatment in a group of patients with an extremely severe prognosis. Keywords: Post-transplant lymphoproliferative disorder, EBV